RHINOSCLEROMA
RHINOSCLEROMA
- Progressive granulomatous disease of the upper respiratory tract.
- Caused by the gram-negative coccobacillus K rhinoscleromatis or Frish Bacillus.
The disease probably begins in areas of epithelial transition such as :
- the vestibule of the nose
- the subglottic area of the larynx
- or the area between the nasopharynx and oropharynx.
- It is a chronic infection of the nose characterised by sclerosis and stenosis of the nasal passage.
- Rhinoscleroma may be found in all age groups, but typically young adults 20–30 years old are most frequently affected.
- Both sexes are equally affected.
- The disease is endemic in several parts of world.
- In India, it is seen more often in northern than in the southern parts.
- Airborne transmission combined with poor hygiene, crowded living conditions, and poor nutrition contributes to its spread.
PATHOLOGY OF RHINOSLEROMA
- The disease is characterized by presence of granulations which infiltrate the submucosa ,with scattered:
- Mikulicz cells (large vacuolated foamy cells containing a central nucleus and acid fast Frisch bacilli),
- Rusell bodies (resmbling plasma cells with an eccentric nucleus and deep eosin staining cytoplasm) and
- lymphocytes in dense fibrous tissue.
CLINICAL FEATURES IN A CASE OF RHINOSCLEROMA
SYMPTOMS
- Blocking of the nose,Nasal Discharge,Headache,Epistaxis
SIGNS
- External nose is firm and woody to touch.
- Anterior Rhinoscopy:Throat:may show extension of the sclerosing process downwards from the nose to the oropharynx,larynx.
- 3 stages:
1. Atrophic Stage: is present initially, with crusting , roomy cavity and atrophic dry nasal mucosa.
2. Granulomatous stage:
- Granulomatous nodules forms in nasal mucosa.
- There is also subdermal infiltration of lower part of external nose & upper lip giving a ‘woody feel’.
- Nodules are painless & non-ulcerative.
3. Cicatricial stage:
- Causes stenosis of nares, distortion of upper lip, adhesions in the nose, nasopharynx & oropharynx.
- May be subglottic stenosis with respiratory distress.
BIOPSY
- Shows infiltration of submucosa with plasma cells, lymphocytes, eosinophils, Mikulicz cells & Russell bodies.
- The latter two are the diagnostic features of the disease.
TREATMENT IN A CASE OF RHINOSCLEROMA
- In rhinoscleromatosis, organism may be difficult to eradicate, despite aggressive therapy. Relapses may occur.
- A combination of conservative surgical debridement and long-term antibiotic coverage is the mainstay of therapy for rhinoscleroma.
- Tetracycline has been shown to be effective .
- Fluoroquinolones may be used as an alternative, given their excellent gram-negative activity and convenient dosing regimen
Exam Important
- Mikulicz cells and Russell bodies are seen in Rhinoscleroma.
- A roomy nasal cavity and atrophic dry nasal mucosa with thick crust formation and woody hard external nose is seen in Rhinoscleroma.
- Rhinoscleroma is caused by Klebsiella Rhinoscleromatis.
- Rhinoscleroma is a slowly progressing granulomatous disease.
- Rhinoscleroma is most common in young adults 20–30 years old.
- Poor nutrition contributes to the spread of Rhinosleroma.
- Drug of choice in Rhinoscleromatosis is Tetracycline.
- Frish bacillus causes Rhinoscleroma.
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